Multiple sclerosis

Background

CNS myelin destruction causes variable motor, sensory, visual and cerebellar dysfunction

Relapsing/remitting (most common)
- Relapse (days-months) followed by remission
Secondary progressive
- Relapses and partial recoveries occur, but disability does not fade away between cycles
Primary progressive
- Symptoms progress slowly and steadily without remission
Progressive relapsing
- Similar to primary progressive but with superimposed flares

Classic patient has multiple presentations for neuro symptoms of different areas of pathology
- Patient often has resolution of the earlier symptoms
Symptoms worsen with increases in body temperature, classically after hot showers (Uhthoff's phenomenon[1])
Muscle/sensory signs:
- Lower extremity weakness usually worse than upper extremity
- Upper motor neuron signs:
  - Hyperreflexia
  - Positive Babinski
- Decrease in proprioception / pain/temperature sensation
- Lhermitte sign
  - Electric shock sensation radiating down back into arms/egs from neck flexion
  - If the discomfort is severe, carbamazepine or gabapentin may be beneficial for some patients.
Optic neuritis
- Initial sign in 30% of patients
- Vision loss (usually unilateral) often preceded by retrobulbar pain
- Blurred vision
- Afferent pupillary defect is pathognomonic for optic neuritis
- Nystagmus
- Diplopia
Internuclear ophthalmoplegia
- Abnormal eye adduction bilaterally and horizontal nystagmus
- Convergence (both eyes center medially) is preserved
Dysautonomia
- Urinary retention (increased risk of UTI/pyelo)
- Constipation or incontinence
- Sexual dysfunction (males)

Neuromuscular weakness
- Upper motor neuron:
  - CVA
  - Hemorrhagic stroke
  - Multiple sclerosis
  - Amyotrophic Lateral Sclerosis (ALS) (UMN & LMN)
- Lower motor neuron:
  - Spinal and bulbar muscular atrophy (Kennedy's syndrome)
- Spinal cord disease:
  - Infection (Epidural abscess)
  - Infarction/ischemia
  - Trauma (Spinal Cord Syndromes)
  - Inflammation (Transverse Myelitis)
  - Degenerative (Spinal muscular atrophy)
  - Tumor
- Peripheral nerve disease:
  - Guillain-Barre syndrome
  - Toxins (Ciguatera)
  - Tick paralysis
  - Diabetic peripheral neuropathy
- NMJ disease:
- Muscle disease:
  - Rhabdomyolysis
  - Dermatomyositis
  - Polymyositis
  - Alcoholic myopathy
Non-neuromuscular weakness
- Can't miss diagnoses:
  - ACS
  - Arrhythmia/Syncope
  - Severe infection/Sepsis
  - Hypoglycemia
  - Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
    - Hypokalemic periodic paralysis
    - Thyrotoxic periodic paralysis
  - Respiratory failure
- Emergent Diagnoses:
  - Symptomatic Anemia
  - Severe dehydration
  - Hypothyroidism
  - Polypharmacy
  - Malignancy
- Other causes of weakness and paralysis
  - Acute intermittent porphyria (ascending weakness)

CSF
- Elevated protein and gamma-globulin (increased oligoclonal bands)
MRI
- Multiple lesions in supratentorial white matter, paraventricular area, spinal cord

MRI brain with contrast of a patient in her mid-20s with new onset MS. Large lesion in left parietal area.
MRI brain with contrast of same patient with new onset MS with another lesion in the left cerebellum.

Hospitalization indicated for:
- Any disease exacerbation associated with significant morbidity
- IV antibiotics or steroid therapy required
- Depression and significant risk of suicide

Flensner G, et al. "Sensitivity to heat in MS patients: a factor strongly influencing symptomology-an explorative survey". BMC Neurol. 2011. 11:27.

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